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Ichthyosis Hystrix

definition

The term has been used to describe several different, rare, severe disorders of keratinisation characterized by massive hyperkeratosis. The term has also been employed to describe localized and linear warty epidermal nevi sometimes associated with mental retardation, seizures or skeletal anomalies. A few exceedingly rare syndromes may be classified as forms of ichthyosis hystrix in the strict sense: - ICHTHYOSIS HYSTRIX GRAVIOR - LAMBERT TYPE, a condition characterized by hyperkeratoses covering the entire body except for the face, genitals, palms and soles. It was first described in the Lambert family in Britain at the beginning of the 18th century. - ICHTHYOSIS HYSTRIX GRAVIOR - CURTH-MACKLIN TYPE, a disorder characterized by hystrix-like changes and keratoses of the palms and soles. The histological findings differ from those of all other ichthyoses. - ICHTHYOSIS HYSTRIX GRAVIOR - RHEYDT TYPE, a condition consisting of keratoses especially on the extremities including the flexor surfaces and to a lesser extent on the face ears. Alopecia and hair and nail abnormalities as well as inner ear deafness were also seen in these patients. Only isolated cases have so far been described of ICHTHYOSIS HYSTRIX GRAVIOR - BAEFVERSTEDT TYPE.